Speakeasy

The patient had a right partial 3rd nerve palsy combined with a mild R 6th nerve palsy, as well as right 5th nerve dysfunction. A right cavernous sinus lesion was suspected. An MRI of brain without contrast and CT head had been done six months ago and had been reported as normal to the patient. Review of those studies revealed some asymmetry to the cavernous sinus area on the right. MRI/MRA brain/orbits without and with gadolinium was then performed. A 2 cm enhancing mass was found in the R pterygopalatine fossa with perineural spread to the cavernous sinus with involvement of R 3rd and 5th cranial nerves. CT scan showed bony erosion and lytic changes involving the R pterygoid base and pterygoid portion of the R sphenoid wing. The patient was then promptly referred to ENT for endoscopic biopsy of the mass via a transnasal approach. Pathologic examination was indicative of Adenoid cystic carcinoma (ACC). The tumor cells were reactive to 34betaE12 while nonreactive to chromogranin and synaptophysin. Two months later, the cancer was resected via a combined ENT/Neurosurgery procedure. This was followed by post-operative XRT (30 treatments). 8 months later, MRI with gadolinium revealed no suspicious area of enhancement to suggest tumor recurrence. Neuro-ophthalmic follow-up revealed a decrease in vision OD to 20/80 due to dry eye and corneal scarring. Color vision was normal. Pupils were 4 and 3.5 mm without RAPD. R face had decreased sensation. Motility of R eye was stable. Frequent lubrication of the R eye was encouraged, and smoking cessation was stressed. ACC, a malignant epithelial cancer, mostly arises in the major and minor salivary glands, nasopharynx, and lacrimal glands. The incidence of intracranial invasion is 4-22%, most commonly by perineural invasion. Prognosis is poor with a survival of less than 50% at 5 yrs.